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[血管母细胞瘤、其特殊形式及罕见部位]

[Angioblastomas, their special forms and rare locations].

作者信息

Winkelmann H

出版信息

Zentralbl Neurochir. 1978;39(2):115-32.

PMID:569953
Abstract

Sorting over of our angioblastoma cases occurring in the period from 1954 to 1977 was carried out under the aspect of special forms and rare localisations. Among a total of 93 angioblastomas in 66 patients, there were 81 infratentorial (60 patients), 5 supratentorial (5 patients), and 7 spinal (4 patients) angioblastomas. For 8 of the infratentorial angioblastomas a participation of the brain stem was found; their prognosis was unfavourable and all these patients died. Angioblastomas occurring in a multiple manner are also involving problems unless they are restricted to the cerebellar spheres and the vermis of the cerebellum. In this group there were 37 angioblastomas occurring in 10 patients with an increased familial occurrence in four cases and a systematic affection with angiomatosis retinae in five patients. Four of these patients also died. The large number of 5 angioblastomas of the cerebrum has induced us to study these cases particularly critically. There was a good prognosis for all these cases; they all were solitary tumours (2 solid tumours and 3 typical large-syctic angioblastomas). The prognosis of the spinal angioblastomas of which 7 were found in 4 patients, is dependent upon location and the question of the systematised affection. Remarkably large is the number of young angioblastoma carriers. All 5 cerebral angioblastomas and 6 cerebellar angioblastomas were found in patients below the age of 18. From these findings conclusions can be drawn with regard to an optimisation of an early detection and the exact angiographic clarificatior in order to avoid unnecessary losses due to transfers being carried out too late and, on the other hand, to sort out inoperable findings.

摘要

对1954年至1977年期间发生的血管母细胞瘤病例,从特殊形式和罕见部位方面进行了分类整理。66例患者共93例血管母细胞瘤,其中幕下81例(60例患者),幕上5例(5例患者),脊髓7例(4例患者)。在8例幕下血管母细胞瘤中发现有脑干受累;其预后不良,所有这些患者均死亡。多发性血管母细胞瘤也存在问题,除非它们局限于小脑半球和小脑蚓部。该组中有10例患者发生37例血管母细胞瘤,4例家族发生率增加,5例患者伴有视网膜血管瘤病。其中4例患者也死亡。大脑出现5例血管母细胞瘤数量较多,促使我们对这些病例进行特别严格的研究。所有这些病例预后良好;它们均为孤立性肿瘤(2例实体瘤和3例典型的大囊型血管母细胞瘤)。4例患者中发现7例脊髓血管母细胞瘤,其预后取决于部位和是否存在系统性受累问题。血管母细胞瘤患者中年轻人的数量相当多。所有5例大脑血管母细胞瘤和6例小脑血管母细胞瘤均在18岁以下患者中发现。根据这些发现,可以就优化早期检测和准确的血管造影诊断得出结论,以避免因转诊过晚而造成不必要的损失,另一方面,筛选出无法手术的病例。

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